About 90% of pilar cysts occur on the scalp, with the remaining sometimes occurring on the face, trunk and extremities. Pilar cysts are significantly more common in females, and a tendency to develop these cysts is often inherited in an autosomal dominant pattern. In most cases, multiple pilar cysts appear at once.
A trichilemmal cyst, also known as a wen, pilar cyst or isthmus-catagen cyst, is a common cyst that forms from a hair follicle. They are most often found on the scalp. The cysts are smooth, mobile and filled with keratin, a protein component found in hair, nails, skin, and horns. They are, however, clinically and histologically distinct from Trichilemmal Horns, which are much more rare and not limited to the scalp.Trichilemmal cysts may run in families and they may or may not be inflamed and tender, often depending on whether or not they’ve ruptured. Rarely, these cysts may grow more extensively and form rapidly multiplying trichilemmal tumors, also called proliferating trichilemmal cysts, which are benign but may grow aggressively at the cyst site. Very rarely, trichilemmal cysts can become cancerous.
Trichilemmal cysts are derived from the outer root sheath of the hair follicle. Their origin is unknown, but it has been suggested that they are produced by budding from the external root sheath as a genetically determined structural aberration. They arise preferentially in areas of high hair follicle concentrations, therefore, 90% of cases occur on the scalp. They are solitary in 30% of cases and multiple in 70% of cases.
Histologically, they are lined by stratified squamous epithelium that lacks a granular cell layer and are filled with compact “wet” keratin. Areas consistent with proliferation can be found in some cysts. In rare cases, this leads to formation of a tumor, known as a proliferating trichilemmal cyst. The tumor is clinically benign, although it may display nuclear atypia, dyskeratotic cells, and mitotic figures. These features can be misleading, and a diagnosis of squamous cell carcinoma may be mistakenly rendered.